This review addresses how anomalous cardiovascular anatomy imparts consequences to the airway, respiratory system mechanics, pulmonary vascular system, and lymphatic system. Abnormal formation or enlargement of great vessels can compress airways and cause large and small airway obstructions. Alterations in pulmonary blood flow associated with congenital heart disease (CHD) can cause abnormalities in pulmonary mechanics and limitation of exercise. CHD can lead to pulmonary arterial hypertension. Lymphatic abnormalities associated with CHD can cause pulmonary edema, chylothorax, or plastic bronchitis. Understanding how the cardiovascular system has an impact on pulmonary growth and function can help determine options and timing of intervention.